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Diagnosing With Dickens

A lonely old man sits at home one Christmas Eve and has a series of unexpected visitors: the restless spirit of his long-dead business partner, followed by the ghosts of Christmas Past, Present and Future. By morning, this wretched, miserly recluse has been transformed into the season’s icon of exuberant celebration. This is, of course, the story of Ebenezer Scrooge — the way Charles Dickens wrote it in his novella “A Christmas Carol.” But there’s another way to tell this familiar tale: an elderly man with no known medical problems has a sudden change in mental status characterized by confusion and vivid hallucinations involving friends and relatives, which leaves him in a state of trembling mania and uncharacteristic euphoria. This also describes Ebenezer Scrooge — but as a doctor might see him. What caused these extraordinary symptoms? a doctor might ask. Could some pathological process have accounted for Scrooge’s remarkable experience?

Dickens has long been recognized as a skillful and accurate chronicler of human behavior. His characters are rendered with a realist’s dedicated, unstinting eye. Physician readers of Dickens’s stories have commented on the precision with which he portrays his characters’ quirks and oddities — many of which are now recognized as disease states. Some of his most memorable characters are virtual case studies of diseases that were not described or understood until long after Dickens’s time.

In “The Pickwick Papers,” Dickens writes about Joe, a young man known for his love of food, generous build and uncanny ability to fall asleep anywhere. In 1956, 120 years after “The Pickwick Papers” began serialization, Dr. C. S. Burwell and his colleagues published a medical case report titled “Extreme Obesity Associated With Alveolar Hypoventilation: A Pickwickian Syndrome.” After quoting Dickens’s description of Joe, the authors go on to describe their patient, a 51-year-old business executive who stood 5 foot 5 and weighed more than 260 pounds.

The patient was suffering from “obesity, fatigue and somnolence.” After several years, he had an experience that “indicated the severity of his disability.” During a weekly poker game, the patient “was dealt a hand of three aces and two kings. ... Because he had dropped off to sleep he failed to take advantage of this opportunity. A few days later he entered ... the hospital.” This was the first medical case report of what is now called sleep apnea, a disorder, usually linked to obesity, in which, during sleep, excess tissue blocks the trachea, or breathing tube, causing multiple awakenings and chronic sleep deprivation.

This was only the first of many medical diagnoses teased from the pages of Dickens’s work. Another character, Mr. Krook, an eccentric shop owner in “Bleak House,” is described in the following way: “He’ll never read. He can make all the letters separately and he knows most of them separately when he sees them ... but he can’t put them together” to make words. This is thought to be the first recorded example of a case of dyslexia — a difficulty that wasn’t recognized as a neurological disorder until nearly 50 years later.

In “A Christmas Carol,” much attention has been paid to Tiny Tim. What did he have that could cripple him, that could kill him, but that could be treated at a time when very few effective therapies were available? The two theories most discussed postulate that he had tuberculosis or a deficiency of vitamin D. Either would have responded to the most likely treatment of the day — a visit to a sanatorium.

Photo

Credit
Illustration by Jan Schwochow

For my part, I have always been fascinated by Scrooge. He is often understood more as a metaphor than as a man, and his transformation is seen as a reflection on the true meaning of Christmas, rather than the physiology of the human body. But Scrooge is drawn with the same meticulous specificity as the other denizens of Dickens’s 19th-century London. Could there be some physical reality underlying this metaphor?

What could have happened to this man that Christmas Eve? What could be behind the revelatory hallucinations of the night? Certainly, Scrooge has his own diagnosis: food poisoning. He tells Marley’s ghost: “You may be an undigested bit of beef, a blot of mustard, a crumb of cheese, a fragment of underdone potato. There’s more of gravy than of grave about you, whatever you are!” Indigestion seems an unlikely culprit — even to the readers of his day — and yet Scrooge makes an interesting point: What if it really were something he ate? Ergotism, poisoning from a mold that grows on damp rye flour, has been known to cause hallucinations and confusion since the dark ages. On the other hand, since entire batches of rye flour were contaminated, ergotism tended to cause epidemics rather than individual cases.

Or were these symptoms caused by an infection? At one point, Scrooge is shaking and sweaty. Is this fear or fever? But what infection could come and go so quickly?

Dementia is the most common cause of a change in mental status among the elderly. Severely demented patients will at times seem to relive experiences from their past, imagining visits and even conversations with friends and family often long dead. But dementia is usually slow and progressive. How could Scrooge continue with the complex tasks of his business if he had Alzheimer’s advanced enough to cause these visions?

Or did Scrooge have a stroke? Apoplexy — as it was known at the time — was a common cause of death and disability. A stroke occurs when a section of brain is suddenly deprived of blood. When this happens in the middle of the brain, it can cause vivid hallucinations. The quality of Scrooge’s hallucinations are consistent with poststroke visions, which are often described as vivid in color and usually occur after dark. And like a stroke, these visions came on suddenly.

In considering these various possibilities, I realized that I needed a second opinion. I turned to Dr. Chance Algar, a careful reader and a recently board-certified neurologist (and my nephew). His initial impression was that a stroke that causes hallucinations will, more than likely, cause physical deficits as well — paralysis or changes in vision or sensation. None of this is apparent in Scrooge: the next morning he leaps out of bed and runs to the window; he sees and speaks without difficulty. From what we can see, Scrooge is no worse off in any way after his terrible ordeal. So what could it be? Were Scrooge’s visions simply a clever literary device, or did they reflect some observed phenomena?

Chance reread the book to broaden his differential diagnosis. He called me the next day, excited. This was dementia, he told me, but not the most common forms — not Alzheimer’s or a dementia caused by multiple small strokes. No, this was a recently described variety known as Lewy body dementia. Symptomatically, this disease lies at the intersection of Alzheimer’s and Parkinson’s disease and was first fully recognized in 1996. Although I saw Scrooge as a man without any medical problems, Chance explained, I had missed some subtle clues in Dickens’s description. The words used to portray Scrooge might apply to many with Parkinson’s disease: expressionless, rigid, nearly immobile. Dickens writes, “The cold within him froze his old features ... and stiffened his gait.” He also has a tremor, a symptom common in Parkinson’s as well as in this strange dementia. But the hallmark of Lewy body disease is the real clincher in this diagnosis: vivid and detailed hallucinations featuring friends and relatives are common. And like Scrooge’s visions, these phantasms are distressing, often terrifying. Finally, in Lewy body dementia, hallucinations occur early in the disease, frequently before the cognitive deficits are apparent. I went back and reread the book with Chance’s diagnosis in mind. It fit. It’s clear that once again Dickens has identified a disease, in this case a full century and a half before medicine did. What then of Scrooge’s miraculous transformation from stingy, miserable wretch to the embodiment of giving and generosity? No disease can account for that. Perhaps that is the true miracle of the story and, maybe, the real meaning of Christmas.